CAJ | 학술논문

目的：探讨磷酸盐尿性间叶肿瘤的临床病理学特征。方法回顾性分析12例磷酸盐尿性间叶肿瘤的临床资料、组织学及免疫表型,并复习相关文献。结果12例中男性8例,女性4例,年龄23~63岁,平均40．5岁；病程1~14年,平均5．6年；患者均有不同程度的骨痛、关节痛和活动受限伴低血磷、高尿磷；肿瘤最大径1~7．5 cm,平均2．7 cm；肿瘤主要由梭形细胞构成,部分病例伴有破骨样多核巨细胞,血管丰富,可见簇状厚壁畸形血管、薄壁血管、散在脂肪岛及软骨样细胞；7例伴有不规则钙盐沉积,2例伴条索状上皮,其中CK(AE1/AE3)阳性；10例核分裂象少见,2例核分裂象多见,其中1例肿瘤细胞异性明显；瘤细胞均表达vimentin、CD56(其中2例分别为弱阳性和灶阳性),11例NSE阳性,8例CD99阳性,7例BCL-2阳性,4例CD34和6例SMA呈不同程度阳性,10例Ki-67增殖指数≤5%,2例分别为10%、25%。随访时间2~108个月,其中2例分别于术后72、84个月复发,其余病例均未见复发。结论磷酸盐尿性间叶肿瘤多表现为良性或低度恶性,其组织学形态多变且缺乏特异性,掌握其共性特征并紧密结合临床才能正确诊断。
목적：탐토린산염뇨성간협종류적림상병이학특정。방법회고성분석12례린산염뇨성간협종류적림상자료、조직학급면역표형,병복습상관문헌。결과12례중남성8례,녀성4례,년령23~63세,평균40．5세；병정1~14년,평균5．6년；환자균유불동정도적골통、관절통화활동수한반저혈린、고뇨린；종류최대경1~7．5 cm,평균2．7 cm；종류주요유사형세포구성,부분병례반유파골양다핵거세포,혈관봉부,가견족상후벽기형혈관、박벽혈관、산재지방도급연골양세포；7례반유불규칙개염침적,2례반조색상상피,기중CK(AE1/AE3)양성；10례핵분렬상소견,2례핵분렬상다견,기중1례종류세포이성명현；류세포균표체vimentin、CD56(기중2례분별위약양성화조양성),11례NSE양성,8례CD99양성,7례BCL-2양성,4례CD34화6례SMA정불동정도양성,10례Ki-67증식지수≤5%,2례분별위10%、25%。수방시간2~108개월,기중2례분별우술후72、84개월복발,기여병례균미견복발。결론린산염뇨성간협종류다표현위량성혹저도악성,기조직학형태다변차결핍특이성,장악기공성특정병긴밀결합림상재능정학진단。
Purpose To study the clinicopathological features of the phosphaturic mesenchymal tumors. Methods The clinicopatho-logical data, histology and immunohistochemical findings of 12 cases of phosphaturic mesenchymal tumors were retrospectively analyzed with review of the relevant literature. Results Among the 12 patients studied, 8 were males and 4 were females. Their age at the time of operation ranged from 23 to 63 years (mean=40. 5 years). The duration of symptoms ranged from 1 to 14 years (mean =5. 6 years) . A history of long-standing bone pain, arthralgia, limitation in movement, accompanied with hypophosphatemia and hyperphos-phaturia were present in all cases. The tumors size ranged from 1 to 7. 5 cm ( mean size=2. 7 cm) . The tumors were primarily com-posed of spindle cells, and were accompanied with osteoclast-like giant cells partly. Their background were rich in blood vessels. In addition, there were scattered tufed thick vascular anomaly, thin vascular, islands of mature adipocytes and chondroid cells. In 7 of the 12 cases, there were dystrophic calcification in an unusual flocculent. Spindle epithelium were noted in 2 cases. Mitotic figures were rare in 10 cases. In 2 of the 12 cases however, mitotic figures were commonly encountered, and in 1 of the 2 cases, heterotypic cells were notablely observed as well. On immunohistochemical study, the tumor cells were all positive for vimentin and CD56. The positivi-ty for NSE, CD99, BCL-2 in 11, 8 and 7 cases were expressed in 12 cases. In 4 and 6 of the 12 cases, CD34 and SMA were positive in various degree. Ki-67 proliferation index were less than 5% in 10 cases, and 10% and 25% in 2 cases. The duration of follow-up ranged from 2 to 108 months ( mean=22 months) . 2 cases were recurred at 72 and 84 months after the operation, respectively. So far, the remaining 10 cases had no recurrence and metastasis. Conclusioin The phosphaturic mesenchymal tumors are either benign or low-grade malignant. Their histomorphology are multivariable and short of specificity. It is important to make the correct diagnosis in combination with their common features and clinical datas.